Echocardiographic characterization of left ventricular apical hypoplasia accompanied by a patent ductus arteriosus
Echocardiographic characterization of left ventricular apical hypoplasia accompanied by a patent ductus arteriosus
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DOI：
10.1093/ejechocard/jeq170
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发表时间：
2011-03-01
影响因子：
--
通讯作者：
Patel, Ayan R.
中科院分区：
文献类型：
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作者：
Haffajee, Jessica A.;Finley, John J.;Patel, Ayan R.
左心室心尖部发育不全是一种罕见的心肌病，其临床过程尚不清楚。在这份报告中，我们描述了一例发生在无症状的50岁男性的心肌病，有手术矫正的动脉导管未闭（PDA）的遥远历史，主要使用经胸超声心动图（TTE）来说明成像特征。该患者因心电图异常被转诊至我院，随后TTE（图1）显示左心室扩张伴中度至重度功能降低;二维和三维定量显示左心室射血分数为30%。左心室呈球形外观，具有薄壁、截短和无动力远端LV。右心室出现拉长，并包裹左心室远端，但右心室收缩功能正常。没有明显的瓣膜异常，也没有残余PDA血流的证据。随后的心脏磁共振（CMR）成像证实了这些发现（图1）。该患者的TTE和CMR结果与LV心尖发育不全一致。到目前为止，这种心肌病仅被描述为一种孤立的先天性异常，主要使用CMR和心脏计算机断层扫描。据我们所知，这是第一个报告的情况下，左心室心尖发育不全与另一种先天性心脏异常，研究结果表明，这种心肌病的独特外观可以很容易地确定与超声心动图。随着越来越多的病例被发现，患者随着时间的推移被随访，这种心肌病的自然病史和最佳治疗方法可能会得到进一步阐明。
Left ventricular (LV) apical hypoplasia is an unusual, recently identified cardiomyopathy, whose clinical course is uncertain. In this report, we describe a case of this cardiomyopathy occurring in an asymptomatic 50-year-old male with a remote history of a surgically corrected patent ductus arteriosus (PDA), primarily using transthoracic echocardiography (TTE) to illustrate the imaging characteristics. This patient had been referred to our institution for an abnormal electrocardiogram, and TTE subsequently (Figure 1) revealed a dilated left ventricle with moderately to severely reduced function; LV ejection fraction was 30% by two- and three-dimensional quantification. The left ventricle had a spherical appearance with a thin-walled, truncated, and akinetic distal LV. The right ventricle appeared elongated and was noted to wrap around the distal left ventricle, but right ventricular systolic function was normal. There were no significant valvular abnormalities, and no evidence of residual PDA flow. Subsequent cardiac magnetic resonance (CMR) imaging confirmed these findings (Figure 1). The TTE and CMR findings seen in this patient are consistent with LV apical hypoplasia. Until now, this cardiomyopathy has been described only as an isolated congenital anomaly primarily using CMR and cardiac computed tomography. To our knowledge, this is the first reported case of LV apical hypoplasia in conjunction with another congenital cardiac abnormality, and the findings demonstrate that the distinctive appearance of this cardiomyopathy can be easily identified with echocardiography. As more cases are recognized and patients are followed over time, the natural history and optimal treatment for this cardiomyopathy may be further elucidated.